A case of refractory chordoma of the clivus with a review of therapeutic targets

Abstract

Chordomas are rare slow-growing tumors of the bone that are locally aggressive and arise from embryonic remnants of the notochord. The clinical presentation of chordomas is dependent on the site of the neoplasm. Brain MRI remains the preferred diagnostic modality imaging modality followed by a tissue biopsy to confirm the underlying pathology and the classical presence of physaliphorous cells. The mainstay of treatment remains surgical resection with adjuvant radiotherapy with a limited role of conventional chemotherapy. For locally advanced tumors, the prognosis remains poor despite multimodality treatment.In this report, we highlight a case of recurrent clival chordomas with disease progression even with several surgical resections and radiotherapy treatment. Our patient presented with intermittent diplopia and further investigation confirmed clival chordoma. The patient was disease-free for 7 years post initial surgical resection and adjuvant radiotherapy but eventually had multiple disease recurrences. We will be discussing ongoing clinical trials in the management of clival chordomas and emerging potential therapies. As with many rare cancers, access to tumor profiling and finding actionable targets through precision medicine, play a crucial role in improving outcomes. It is imperative that further studies and clinical trials are instituted to influence clinical outcomes.

Keywords

• Chordoma
• Immune checkpoint inhibitors
• Gamma-knife radiosurgery
• Tumor profiling
• Clivus